9 But he said to me, “My grace is sufficient for you, for my power is made perfect in weakness.” Therefore I will boast all the more gladly about my weaknesses, so that Christ’s power may rest on me.
I have Corticobasal Syndrome or C.B.S. Formerly, this would have been named Corticobasal Ganglionic Degeneration or C.B.G.D. Today, Corticobasal Syndrome is the handle given to this illness during the life of the patient because it has to be confirmed pathologically (with an autopsy of the brain tissue) after the passing of the patient. All Parkinson's Plus Syndromes, Multiple System Atrophy, Progressive Supranuclear Palsy, Lewy Body Dementia and Corticobasal Syndrome are not diagnosed, absolutely, until a brain study can be completed. The diagnosis is called "probable" or "possible" during the life of the patient.
So, after 11 years of treatment for Parkinson's Plus-- thought to be probably Multiple System Atrophy (MSA), it has been determined that I do have M.S.A. and also Corticobasal Syndrome. The reasons are that I have the movement disorder and cognitive symptoms of C.B.S., but I also have the neurologically caused autonomic failure of M.S.A. I have explained this autonomic failure on this blog before and you can refer back to it in my pages above, the links to previous posts to the right or by reading my book, I Will Go On: Living with a Movement Disorder. In this post, I will now explain the basics of Coriticobasal Syndrome.
Overview of Corticobasal Syndrome
Corticobasal Syndrome is a movement disorder that is similar to Parkinson's Disease, though faster progressing and includes additional symptomatic issues that are more complicated earlier in the disease process than in Parkinson's Disease. Parkinson's Disease is similar because it has symptoms known as parkinsonism, which include: 1) tremor, 2)balance problems, 3)stiffness and 4)slowness -- all caused by a degeneration of brain neurons. Because Coriticobasal Syndrome also involves the Basalganglia (movement control center of the brain and is also involved in Parkinson's) some of the symptoms overlap, thus it is a Parkinson's Plus syndrome.
That is where the similarity stops. I do not have Parkinson's and I never have. From the beginning, my neurologist at the time said, "This is so much more than Parkinson's Disease."
It was thought to be either Progressive Supranuclear Palsy or Shy Drager (an earlier name given to the condition now referred to as Multiple System Atrophy). Progressive Supranuclear Palsy is a close cousin to Coricobasal Syndrome, so we have been in the right arena over this past decade. The treatment and care given to me were helpful and would have been the same with either designation. I would have had the Brain Surgery I had in July 2012 and it would have been considered helpful and effective in giving me better movement and more ability to walk with assistance, rather than being in a wheelchair most of the time, as I had been prior to the Deep Brain Stimulation Surgery.
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After brain surgery to implant stimulators in two places in my head, with a brain pacemaker in my chest, I was able to resume playing in the church band for several years along with my son, Mark, shown here on the keys.
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Corticobasal Syndrome is a movement disorder that includes walking trouble, tremors/jerky movements, eye control movement impairment, swallowing difficulty, rigidity and balance trouble. It also causes Cognitive Changes that affect behavior, speech, mood problems and difficulty planning and concentrating. It is a form of dementia called, Frontotemporal Dementia, but it is not the same disease as other forms of dementia, such as Alzheimer's Disease (A.D.). It should not be confused with A.D.
What Causes Corticobasal Syndrome?
It is not clear what causes the degenerative process in the brain of individuals with C.B.S., but what we do know is that there is a specific protein in the brain known as Tau that breaks down creating tangles. These tangles destroy nerve pathways and inhibit the ability of the brain to communicate signals that help us move-- whether it be coordinating movements, walking, talking, grasping, swallowing or seeing.
What is Alien Limb Syndrome?
One of the symptoms of C.B.S. is a phenomena called alien limb syndrome, where a hand, arm, or leg seems to have a mind of its own, assuming postures and movements that seem alien to the patient's awareness. Also, there are twisted postures of hands, fingers, arms, neck and/or legs/feet. These movements may also appear in a jerky unpredictable manner, while a form of tremor in some, it may be less regular and random in its form. Parkinson's Disease typically, when tremor exists (not all Parkinson's patients have a tremors) it is a slow, regularly patterned tremor that occurs generally during rest.
What is a Taupathy?
The illnesses that are caused by the breakdown of the protein Tau, include: Alzheimer's Disease and Progressive Supranuclear Palsy, along with Corticobasal Syndrome.
How is it treated?
C.B.S. does not have a cure, but there are a number of treatments that may help with symptom relief. Some of the medicines used for Parkinson's are also given to those with C.B.S., which has been true in my case. Sinemet is given to help some with rigidity and tremor. I do receive some benefit from Sinemet. Other treatments address the cognitive issues and can include medicines used to assist with memory. Speech therapy can be used to assist with swallowing problems, which has been beneficial in my case. Swallowing problems have to be combated because they can lead to serious choking and eventual pneumonia. Falling and gait difficulty require physical therapy and assistive devices such as canes, walkers, wheelchairs and electric scooters. I use all of the above. Special lenses may be prescribed to help with the eye movement control problems. These would be meant to help to prevent double vision. This eye aiming and movement problem occurs in both Progressive Supranuclear Palsy and Corticobasal Syndrome. These two syndromes are often confused with one another and a brain study post-mortem is the only way to distinguish which is the correct diagnosis. Both will show Tau tangles in the brain tissue.
In Summary
Understand that my explanation is from previous study and is by no means a scientific description. Rather, think of it as a start toward me helping my family and friends gain knowledge about that with which what I am struggling. I see this as an opportunity to overcome these symptoms and disabling problems to whatever degree is possible through continuing effort and positive forward motion in life. This is a daily decision and a better choice than sitting down and giving up. Along with C.B.S., I am fighting the low body temperature regulation problems, the falling blood pressure (orthostatic hypotension), trouble with urination, digestive dysfunction, breathing difficulty, heartbeat irregularities-- all of which are caused by a breaking down of neurons from a different cause than C.B.S. M.S.A. involves the folding of alpha synuclein protein, rather than Tau tangles. It is being found that some patients have both proteins causing neurodegeneration. Diseases aren't fair and don't "leave you alone" because you already struggle with another condition. There is no rule than you can't get two neurodegenerative diseases. (This link will take you to an article that gives an example of how these two proteins misfolding can work together in the disease process: https://mayoclinic.pure.elsevier.com/en/publications/alpha-synuclein-and-tau-teammates-in-neurodegeneration
Thank you for taking time to read this explanation. Hopefully, this will help to explain the differences that make my condition an exception to the more common brain illness known as Parkinson's Disease. Henceforth, I will hope to take part in the effort to bring light to this serious condition known as Corticobasal Syndrome. I am very thankful for the exceptional Movement Disorder Specialist I am treated by who investigated my brain disease very thoroughly and with all relevant technology and advanced diagnostic techniques. Special thanks, as always, to my caregiving spouse, Karrie, who loves me and shows such grace and patience in dealing with all of the inabilities and changes that affect her nearly as much as me. Also, I thank my sons, their spouses and our grandchildren, who are with me frequently, and are helpful and encouraging to me in making the most of every day. -- Patient-Online